A Case Report on Congenital Nasal Pyriform Aperture Stenosis

Abstract

Introduction: Congenital nasal pyriform aperture stenosis is a rare cause of neonatal respiratory distress.
 Observation: A female neonate was born by cesarean section at 39 SA for hydramnios, triple scarred uterus and macrosomia. He was macrosomic and had no facial dysmorphia, he presented at H1 of life with respiratory distress and noisy mouth breathing. He cyanosed with each feeding attempt. On facial CT, the choanae were patent with a median central incisor and piriformis orifice stenosis. Nasal breathing gradually normalized. The hospitalization lasted 8 days with a good evolution.
 Discussion: Congenital piriformis stenosis is a rare cause of congenital nasal obstruction. The association with median incisor syndrome is not exceptional. Diagnosis must be made early for proper management. Cyanosis and false airway can be life threatening. Associated abnormalities should be investigated by CT and MRI. Treatment is variable and may be surgical if the stenosis is severe. The prognosis is good in the absence of severe malformations or associated mental retardation.
 Conclusion: In case of neonatal respiratory distress, the Congenital pyriformis stenosis must be among the causes, a rapid diagnostic assessment and management are necessary.